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Phenotypes Associated with This Genotype
Genotype
MGI:5908182
Allelic
Composition
Tg(Myh6-Mtpn)4Ssen/0
Genetic
Background
involves: FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-Mtpn)4Ssen mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• hypertrophy eventually leads to heart failure at around 36 weeks of age

growth/size/body
• heart weight/body weight ratio is increased
• mice develop progressive cardiac hypertrophy

cardiovascular system
• large nuclei in myocytes, indicating myocyte hypertrophy
• cross-sectional area of myocytes in 4 week old and 9 month old mice progressively increases, indicating hypertrophy
• however, no myocyte atrophy is seen
• multiple foci of classic myocyte disarray are seen in heart tissue
• atria are enlarged by 4 weeks of age
• both right and left ventricles are enlarged and display increased septal thickness
• heart weight/body weight ratio is increased
• mice develop progressive cardiac hypertrophy
• the left ventricle heart walls are severely thickened at 18 weeks of age and show concentric hypertrophy
• ventricles are enlarged by 4 weeks of age
• fibrotic foci are seen in hearts
• dystrophic calcification in hearts
• mice exhibit decreased fractional shortening at 9 months of age
• echocardiography shows increased left atrial diameter, intraventricular septal wall thickness and left ventricular posterior wall thickness, with no change in fractional shorting at 4 weeks of age
• echocardiography shows hypertrophied septum, enlarged left ventricular diastolic and systolic dimensions, lower fractional shortening, a trend toward left atrial diameter enlargement, and increased left ventricular posterior wall thickness at 9 months of age
• mice develop symptoms of heart failure, including lethargy, edema, pulmonary effusion and lack of alertness

behavior/neurological
• at end stage of disease

homeostasis/metabolism
• at end stage of disease
• large amount of pleural effusion is seen in 9 month old mice

muscle
• large nuclei in myocytes, indicating myocyte hypertrophy
• cross-sectional area of myocytes in 4 week old and 9 month old mice progressively increases, indicating hypertrophy
• however, no myocyte atrophy is seen
• multiple foci of classic myocyte disarray are seen in heart tissue
• mice exhibit decreased fractional shortening at 9 months of age

respiratory system
• large amount of pleural effusion is seen in 9 month old mice

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
congestive heart failure DOID:6000 J:89823
hypertrophic cardiomyopathy DOID:11984 J:89823


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
01/24/2023
MGI 6.22
The Jackson Laboratory